Assessing the role of glycosphingolipids in the phenotype severity of Fabry disease mouse model

Assessing the role of glycosphingolipids in the phenotype severity of Fabry disease mouse model

Fabry disease is caused by deficient activity of α-galactosidase A, an enzyme that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins, and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), globotriaosylsphingosine (lyso-Gb3), and galabio...

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Bibliographic Details
Main Authors: Siamak Jabbarzadeh-Tabrizi, Michel Boutin, Taniqua S. Day, Mouna Taroua, Raphael Schiffmann, Christiane Auray-Blais, Jin-Song Shen
Format: Article
Language:English
Published: Elsevier 2020-11-01
Series:Journal of Lipid Research
Subjects:
glycolipids
sphingolipids
inborn errors of metabolism
storage diseases
globotriaosylceramide
globotriaosylsphingosine
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520437290

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http://www.sciencedirect.com/science/article/pii/S0022227520437290

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